Cortico-basal degeneration is a rare degenerative disease connected with Tau protein pathology. Epidemiology of cortico-basal degeneration is unknown. The authors present a case of 59 years old woman with suspicion of cortico-basal degeneration. The extrapyramidal symptoms mainly on the right side with "alien limb phenomenon" and dystonia of lower limb is observed in our patient. Cortico-subcortical brain atrophy was present in MRI scans. EEG was asymmetrical. No improvement was noticed after L-Dopa. Treatment of amantidine caused the transient improvement.